Plasma chromogranin A in patients with multiple endocrine neoplasia type 1

Plasma chromogranin A (CgA) has been claimed to be a sensitive marker for n euroendocrine tumors, but its role in the early diagnosis of multiple endoc rine neoplasia type 1 (MEN 1) pancreatic endocrine tumors has not been eval uated. We measured CgA in 36 patients with MEN 1, of whom 9 lacked pancreat ic involvement, 20 had biochemical evidence of pancreatic endocrine tumors, and 7 displayed radiologically detectable pancreatic tumors. CgA was also analyzed in 25 patients with sporadic pancreatic endocrine tumors, 39 subje cts with inflammatory bowel disease, 7 patients harboring nonendocrine panc reatic disease, and 19 healthy controls. Four of 9 of the MEN 1 patients wi thout pancreatic involvement had elevated CgA. Furthermore, 60% with bioche mically unequivocal tumors and all with a radiologically visible tumor show ed elevations. All 25 patients with sporadic pancreatic endocrine tumor had increased CgA, as had 28% of patients with inflammatory bowel disease and 57% with nonendocrine pancreatic disease. Mean day to day CgA variation was 29% (range, 0-113%) in the neuroendocrine tumor patients and 21.0% (range, 0.0-47%, within reference range) among healthy controls. In summary, nonen docrine diseases may cause elevation of CgA, and its spontaneous variation can be considerable. Plasma chromogranin A is the most sensitive of the bas al markers for neuroendocrine tumors, but cannot replace other established measures when screening for early pancreatic involvement in MEN 1.